How Is Pulmonary Hypertension Treated?

Pulmonary hypertension (PH) has no cure, but treatment may help relieve symptoms and slow the progress of the disease.

PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and how severe it is. (For more information, see "Types of Pulmonary Hypertension.")

Group 1 Pulmonary Arterial Hypertension

This type of PH includes PH that's inherited, that has no known cause, or that's caused by certain conditions. Treatment for group 1 pulmonary arterial hypertension (PAH) includes medicines or medical procedures.

Medicines

If you have group 1 PAH, you may need medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.

Examples of these medicines include phosphodiesterase-5 inhibitors, prostanoids, endothelin receptor antagonists, and calcium channel blockers.

To find out which of these medicines will work best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. This test is done during right heart catheterization.

Medical and Surgical Procedures

If you have group 1 PAH, you may need one or more of the following procedures.

Atrial septostomy. For this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the two upper chambers of your heart). This wall is called the septum.

A tiny balloon on the tip of the tube is inflated to create an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow. Atrial septostomy is rarely done in the United States.

Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.

Heart–lung transplant. A heart–lung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.

Group 2 Pulmonary Hypertension

Group 2 PH is caused by conditions that affect the left side of the heart, such as mitral valve disease. Treating these conditions also will help treat PH. Treatments may include lifestyle changes, medicines, and surgery.

Group 3 Pulmonary Hypertension

Group 3 PH is linked to lung conditions, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease. Group 3 PH also may be linked to certain sleep disorders, such as sleep apnea.

If you have this type of PH, you may need oxygen therapy. This treatment raises the level of oxygen in your blood. You'll likely get the oxygen through soft, plastic prongs that fit into your nose. You can get oxygen therapy at home or in a hospital.

Your doctor also may recommend other treatments if you have an underlying lung disease.

Group 4 Pulmonary Hypertension

This type of PH is due to blood clots in the lungs, blood clotting disorders, or sickle cell anemia. If you have this type of PH, your doctor will likely prescribe anticoagulants, or "blood thinners." These medicines prevent clots from forming or from getting larger.

In some cases, surgery is used to remove scarring in the pulmonary arteries due to old blood clots.

Group 5 Pulmonary Hypertension

Various diseases and conditions cause group 5 PH. Examples include sarcoidosis, Langerhans cell histiocytosis, and lymphangioleiomyomatosis (LAM). This type of PH also may be due to an object, such as a tumor, pressing on the pulmonary blood vessels.

This type of PH is treated by treating its cause.

All Types of Pulmonary Hypertension

Several treatments may be used for all types of PH. These treatments include:

• Diuretics, or "water pills." These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
• Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
• Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin is sometimes used to control the heart rate when certain abnormal heart rhythms occur, such as atrial fibrillation or atrial flutter.
• Oxygen therapy. This treatment raises the level of oxygen in your blood.
• Physical activity. Regular activity may help improve your ability to be active. Your doctor can help you create an exercise plan that's safe for you.

Research on new treatments for PH is ongoing. These treatments offer hope for the future. Talk to your doctor about whether you should consider participating in research studies.